Matches in SemOpenAlex for { <https://semopenalex.org/work/W2274031004> ?p ?o ?g. }
- W2274031004 endingPage "17" @default.
- W2274031004 startingPage "9" @default.
- W2274031004 abstract "Long QT syndrome type 1 (LQT1) is caused by mutations in KCNQ1 coding slowly-activating delayed-rectifier K+ channels. We identified the novel missense mutation M437V of KCNQ1 in a LQT1 patient. Here, we employed iPS cell (iPSC)-derived cardiomyocytes to investigate electrophysiological properties of the mutant channel and LQT1 cardiomyocytes. To generate iPSCs from the patient and a healthy subject, peripheral blood T cells were reprogrammed by Sendai virus vector encoding human OCT3/4, SOX2, KLF4, and c-MYC. Cardiomyocytes were prepared from iPSCs and human embryonic stem cells using a cytokine-based two-step differentiation method and were subjected to patch clamp experiments. LQT1 iPSC-derived cardiomyocytes exhibited prolongation of action potential duration (APD), which was due to a reduction of the KCNQ1-mediated current IKs; Na+, Ca2+ and other K+ channel currents were comparable. When expressed in HEK293 and COS7 cells, the mutant KCNQ1 was normally expressed in the plasma membrane but generated smaller currents than the wild type. Isoproterenol significantly prolonged APDs of LQT1 cardiomyocytes, while shortening those of healthy ones. A mathematical model for IKs-reduced human ventricular myocytes reproduced APD prolongation and generation of early afterdepolarizations (EADs) under β-adrenergic stimulation. QT prolongation of the LQT1 patient with the mutation M437V of KCNQ1 was caused by IKs reduction, which may render the patient vulnerable to generation of EADs and arrhythmias." @default.
- W2274031004 created "2016-06-24" @default.
- W2274031004 creator A5007562144 @default.
- W2274031004 creator A5010491253 @default.
- W2274031004 creator A5020837304 @default.
- W2274031004 creator A5030805961 @default.
- W2274031004 creator A5035616439 @default.
- W2274031004 creator A5036276159 @default.
- W2274031004 creator A5043612854 @default.
- W2274031004 creator A5043809808 @default.
- W2274031004 creator A5045038201 @default.
- W2274031004 creator A5065064934 @default.
- W2274031004 creator A5068706107 @default.
- W2274031004 creator A5069453818 @default.
- W2274031004 creator A5079582513 @default.
- W2274031004 creator A5089636709 @default.
- W2274031004 date "2016-06-01" @default.
- W2274031004 modified "2023-10-18" @default.
- W2274031004 title "Electrophysiological properties of iPS cell-derived cardiomyocytes from a patient with long QT syndrome type 1 harboring the novel mutation M437V of KCNQ1" @default.
- W2274031004 cites W1549569049 @default.
- W2274031004 cites W154975620 @default.
- W2274031004 cites W1575472445 @default.
- W2274031004 cites W1971974734 @default.
- W2274031004 cites W1974273055 @default.
- W2274031004 cites W1980463871 @default.
- W2274031004 cites W1996802734 @default.
- W2274031004 cites W2002943691 @default.
- W2274031004 cites W2009678592 @default.
- W2274031004 cites W2011032223 @default.
- W2274031004 cites W2015441097 @default.
- W2274031004 cites W2020552191 @default.
- W2274031004 cites W2030816979 @default.
- W2274031004 cites W2034867162 @default.
- W2274031004 cites W2035491306 @default.
- W2274031004 cites W2050996123 @default.
- W2274031004 cites W2068437492 @default.
- W2274031004 cites W2077113957 @default.
- W2274031004 cites W2087731349 @default.
- W2274031004 cites W2102389881 @default.
- W2274031004 cites W2104996733 @default.
- W2274031004 cites W2108022643 @default.
- W2274031004 cites W2115644752 @default.
- W2274031004 cites W2115765852 @default.
- W2274031004 cites W2116018623 @default.
- W2274031004 cites W2133808555 @default.
- W2274031004 cites W2135227689 @default.
- W2274031004 cites W2158697417 @default.
- W2274031004 cites W2165970697 @default.
- W2274031004 cites W2165992501 @default.
- W2274031004 doi "https://doi.org/10.1016/j.reth.2015.12.001" @default.
- W2274031004 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6581809" @default.
- W2274031004 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/31245483" @default.
- W2274031004 hasPublicationYear "2016" @default.
- W2274031004 type Work @default.
- W2274031004 sameAs 2274031004 @default.
- W2274031004 citedByCount "12" @default.
- W2274031004 countsByYear W22740310042017 @default.
- W2274031004 countsByYear W22740310042019 @default.
- W2274031004 countsByYear W22740310042020 @default.
- W2274031004 countsByYear W22740310042021 @default.
- W2274031004 countsByYear W22740310042022 @default.
- W2274031004 crossrefType "journal-article" @default.
- W2274031004 hasAuthorship W2274031004A5007562144 @default.
- W2274031004 hasAuthorship W2274031004A5010491253 @default.
- W2274031004 hasAuthorship W2274031004A5020837304 @default.
- W2274031004 hasAuthorship W2274031004A5030805961 @default.
- W2274031004 hasAuthorship W2274031004A5035616439 @default.
- W2274031004 hasAuthorship W2274031004A5036276159 @default.
- W2274031004 hasAuthorship W2274031004A5043612854 @default.
- W2274031004 hasAuthorship W2274031004A5043809808 @default.
- W2274031004 hasAuthorship W2274031004A5045038201 @default.
- W2274031004 hasAuthorship W2274031004A5065064934 @default.
- W2274031004 hasAuthorship W2274031004A5068706107 @default.
- W2274031004 hasAuthorship W2274031004A5069453818 @default.
- W2274031004 hasAuthorship W2274031004A5079582513 @default.
- W2274031004 hasAuthorship W2274031004A5089636709 @default.
- W2274031004 hasBestOaLocation W22740310041 @default.
- W2274031004 hasConcept C104317684 @default.
- W2274031004 hasConcept C107459253 @default.
- W2274031004 hasConcept C118441451 @default.
- W2274031004 hasConcept C126322002 @default.
- W2274031004 hasConcept C145103041 @default.
- W2274031004 hasConcept C146403970 @default.
- W2274031004 hasConcept C147944092 @default.
- W2274031004 hasConcept C155730078 @default.
- W2274031004 hasConcept C174510640 @default.
- W2274031004 hasConcept C185263204 @default.
- W2274031004 hasConcept C185592680 @default.
- W2274031004 hasConcept C207200792 @default.
- W2274031004 hasConcept C2779703243 @default.
- W2274031004 hasConcept C501734568 @default.
- W2274031004 hasConcept C54355233 @default.
- W2274031004 hasConcept C71924100 @default.
- W2274031004 hasConcept C75563809 @default.
- W2274031004 hasConcept C81885089 @default.
- W2274031004 hasConcept C86803240 @default.
- W2274031004 hasConcept C95444343 @default.
- W2274031004 hasConceptScore W2274031004C104317684 @default.